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Effect of stem cell factor on colony growth from acquired and
constitutional (Fanconi) aplastic anemia
GP Bagnara, P Strippoli, L Bonsi, MF Brizzi, GC Avanzi, F Timeus, U Ramenghi, G Piaggio, J Tong and M Podesta
Divisione di Ematologia, Ospedale San Martino, Genova, Italy.
The aim of this study was to evaluate the effect of stem cell factor (SCF)
on the in vitro growth of bone marrow hematopoietic progenitors from
patients with acquired severe aplastic anemia (AA) or Fanconi's anemia
(FA). For this purpose, we studied 11 patients with acquired AA (5 at
diagnosis, 6 after ALG treatment), 12 patients with FA, and nine normal
controls. Bone marrow cells were plated in vitro for colony- forming unit
granulocyte-macrophage (CFU-GM) (in the presence of granulocyte-macrophage
colony-stimulating factor [GM-CSF]), and for burst-forming unit-erythroid
(BFU-E) and CFU-granulocyte, erythroid, monocyte, megakaryocyte (CFU-GEMM)
colonies (in the presence of erythropoietin and interleukin-3 [IL-3]), with
or without 20 ng/mL of SCF. In normal controls, SCF enhanced the growth of
CFU-GM colonies from 103 to 263 (median), of BFU-E from 168 to 352, and of
GEMM colonies from 6 to 38/10(5) cells plated. In patients with acquired
AA, SCF induced a significant enhancement of BFU-E growth (8 to 29; P =
.01) and allowed the formation of GEMM colonies that were not scored in
baseline culture conditions (0 to 8; P = .01). CFU-GM growth was enhanced
(4 to 20), but not significantly (P = .3). This was true both for patients
at diagnosis and after antilymphocyte globulin treatment. By contrast, 10
of 12 FA patients grew no CFU-GM, BFU-E, or CFU-GEMM colonies, with or
without SCF. In two FA patients (one transfusion- dependent and one
transfusion-independent), an enhancement of CFU-GM and/or BFU-E was
observed. The lack of response of hematopoietic progenitor cells from FA
patients to GM-CSF+SCF or IL-3+SCF was not dependent on a defective
expression of cytokine receptor messenger RNAs. Northern blot analysis
showed in marrow cells from acquired AA and FA patients the presence of
normal transcripts for alpha- and beta- chains of GM-CSF/IL-3 receptor and
for c-kit protein. In conclusion, SCF promotes the in vitro growth of
hematopoietic progenitors in patients with acquired AA, but not in patients
with FA, pointing out the intrinsic nature of the defect in the latter
disorder.
Volume 80,
Issue 2,
pp. 382-387,
07/15/1992
Copyright © 1992 by The American Society of Hematology
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